Marfan Syndrome

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Hey guys, In this lesson we are going to go over the basics about Marfan Syndrome.

Marfan Syndrome is a genetic disorder that causes abnormalities in connective tissue. Essentially there is a mutation on something called the fibrillin gene. This gene plays a really important role in forming connective tissue. Without it patients have problems with their musculoskeletal system, their cardiac system the eyes, and skin.

I made this table so you can quickly look at the various symptoms. Probably the most noticeable symptom is a tall, thin body with very long arms, legs and fingers. A positive wrist sign, which you can see in the photo here demonstrates how long their limbs and fingers are. Chest deformities are common and their joints are hypermobile. They are also very prone to having severe scoliosis.

In the cardiac system the major issues are the valves and in the aorta. Mitral valve prolapse, aortic enlargement and aortic aneurysm. The problems with the aorta increase their risk for having an aortic dissection, which is a tear in the aorta that is often fatal.

Stretch marks are common on the skin and in the eyes the lens can be dislocated. Nearsightedness is common and so is glaucoma.
The top priority for management is to keep a very close eye on the child’s cardiac status. They may need a valve replacement and they need to be aware of signs of an aortic dissection (the most distinct symptom is sudden severe chest pain that feels like tearing or ripping).

To reduce the risk of damage to the aorta these patients are often prescribed a beta blocker. This helps by reducing cardiac after load so there is less pressure on the aorta.

Another important part of their management is to treat the scoliosis. Again, they usually have severe scoliosis so surgery is usually needed.

Most people with Marfan Syndrome can life a full life with a normal life expectancy so long as their cardiac function is very carefully monitored and efforts are put in place to minimize stress on the aorta. One big part of this prevention is making sure that these kids avoid activities that will strain their heart and joints.

Your priority nursing concepts for a patient with Marfan Syndrome are mobility and human development.
Okay, lets to over your key points for this lesson. Marfan syndrome is a genetic disorder that affects connective tissue. It primarily causes problems with the musculoskeletal system, cardiac system, eyes and skin.

For the cardiac system it can cause mitral valve prolapse and there's an increased risk for aortic aneurysm and aortic dissection. Beta blockers are commonly prescribed to reduce stress on the aorta.

They will likely need surgical treatment for scoliosis.

Patient education is super important so they know to avoid activities that will put stress on the heart and joints.

That's it for our lesson on Marfan Syndrome. Make sure you check out all the resources attached to this lesson. Now, go out and be your best self today. Happy Nursing!
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